Indian childhood cirrhosis (ICC) is an autoimmune disorder affecting children in India characterized by fever, abdominal distension, and hepatosplenomegaly. It is most common in male children ages 6 months to 4 years from low-income families in rural areas. The exact cause is unknown but is likely due to a combination of genetic susceptibility and environmental factors like copper toxicity, infections, and malnutrition. Clinically, ICC presents with abdominal distension, fever, jaundice, and eventually liver failure if not treated. Diagnosis involves liver function tests and biopsy showing cirrhosis. Treatment focuses on nutrition, antibiotics, medications to reduce copper levels, and managing complications. Nursing care emphasizes rest, diet, intravenous fluids

1. INDIAN CHILDHOOD CIRRHOSIS
Presented by
Ms Arifa T N,
Second year M.Sc Nursing, MIMS
CON

2. INTRODUCTION
• Indian child hood cirrhosis (ICC) of liver is a very serious
condition affecting children in Indian subcontinent.
• The word Cirrhosis derived from the Greek word ‘scirrbus’ , in
which the surface of the liver appears orange or tawny at
autopsy
• It is one of the non-Wilsonian copper overload hepatic disorder.
• The first case in India was reported in the year 1880 in Kolkata.

3. • Over 90% cirrhosis in pediatric age group is accounted by what are
now called the Indian childhood cirrhosis and Jamaican cirrhosis or
veno-occlusive disease (VOD) of liver.

4. Definition
ICC is an autoimmune disorder characterized by fever,
abdominal distension and hepatosplenomegaly.
It is a progressive disease with abdominal distension,
marked irritability, unexplained irregular low-grade fever,
hepatosplenomegaly with hepatic failure, ascites and
jaundice.

6. Epidemiology and incidence
• Prevalent all over India in some particular communities.
▫ South India—Brahmins
 Central India—Banias
 North India—Banias and Jats
 North Indian Hills—Rajputs
• Male children are 4 times more sufferer than female (4:1).
• First born children are at risk.
• Between the ages of 6months and 4 years.

7. • Family predisposition for ICC, siblings and twins are affected
• Low middle class vegetarian family of rural area
• Predisposition with disorders like peptic ulcer, asthma, and migraine.
• Now, decrease in incidence of ICC.
• Use of copper and brass vessels for cooking

8. Etiology
• The exact etiology is not known
• Predisposing factors
▫ Toxic (copper intoxication)
 Presence of copper binding protein (orcein) in the liver
 Supplementary milk feed before 3 months
 Use of copper and copper alloy vessels for cooking
▫ Viral infections
 Infective hepatitis during neonatal period
▫ Immunological factors
 high levels of circulating immunocomplexes insult hepatocytes
causes immune mediated injury to liver

9. ▫ Nutritional factors
 Malnutrition during young age
▫ Hepatotoxic agents
 Aflatoxin (Aspergillus flavus) that grows on groundnuts, maize, and rice.
▫ Family history
 Hall mark of ICC
 An increased prevalence of peptic ulcer, asthma, diabetes and migraine
▫ Metabolic factors
 Inborn error of tryptophan metabolism, aminoaciduria, aminoacidaemia,
disturbed lactose, zinc, copper and magnesium metabolism
▫ Super added factors for genetically prone children
 Viral, toxic, metabolic and autoimmune

10. Pathophysiology
Etiological factors contribute to marked damage to the hepatocytes leading to complete
disorganization of liver architecture.
Make changes in size, colour (gray to frank green ) and formation macro –micro nodules on
the surface of the liver
Absence of regenerative changes in the liver leads to degeneration, necrosis and fibrosis
of hepatic lobules leading to cirrhosis
Kupffer’s cells show mild degree of proliferation. The necrosis of hepatocytes with
ballooning, Mallory hyaline pericellular intralobular fibrosis and inflammatory cell and
cholestasis

11. Clinical features
• Insidious or acute onset
Insidious
onset
Precirrhotic
symptoms
The cirrhotic
symptoms
Stage I
Stage II
Stage III
Acute onset

12. Insidious onset
• Precirrhotic symptoms
▫ Irritability
▫ Disturbed appetite
▫ Chalky, pasty stools and distension of abdomen
▫ Constipation or diarrhea
▫ Often sight irregular fever

13. • Cirrhotic symptoms
▫ Stage I
 Slight fever
 Liver is enlarged up to 3-5 cm, edges become sharp, giving a
leafy boarder appearance
 Children exhibit jaundice
 Poor growth
 Anorexia
 Constipation or diarrhea
 Clay colored stools
 Growth failure

14. ▫ Stage II
 Diffuse hepatomegaly
 Splenomegaly
 Ascites
 Oesophageal varices
 Haemoptysis
 Anaemia
 Muscle weakness
 Lethargy
 GI bleeding

15. ▫ Stage III
 It is the terminal stage of the disease
 Restlessness
 Confusion
 Dyspnea and cyanosis on exertion
 Evidence of hepatocellular failure in the form of palmar
erythema ans spider nevus appearance on the upper torso.
 A peculiar garlic odor is present in patients with impending
liver cell failure
 Enlarged and hard spleen
 Terminally, there is jaundice and hepatic coma and is often
associated with gastrointestinal bleeding
 The child may die at this stage either from hepatic failure or
intercurrent infections.

17. Acute onset
• Sudden onset of disease
• Sometimes asymtomatic for a variable period and then
shows the manifestations of insidious onset.
▫ Sudden onset of fever
▫ Jaundice
▫ Clay colored stools
▫ Hepatospenomegaly
▫ Death due to hepatic coma

18. Diagnostic evaluation
• Perform history collection
• Complete physical examination
▫ Liver can be palpable
▫ Very firm in consistency and its boarders will be sharp
▫ Hepatic bruit on auscultation
▫ In case of ascites, fluid thrill test can be done
• Liver function tests
▫ Increased ALT (Alanine Transaminase)
▫ Increased GGT (γ-Glutamyl Transpeptidase)
• Prothrombin time (PT) (prolonded), clotting time and bleeding time
should be assessed
• Liver biopsy
▫ Sclerosis of liver
• Cupriuresis: It involves testing the presence of copper in urine after
administration of D-pencillamine

19. Multidisciplinary management
• If diagnosed in early stage it can be treated
• Initial stage
▫ Diet :
 Good quality proteins, minerals and vitamines
▫ Antibiotics : infection/infestations
▫ D-pencillamine 20-40 mg/kg per day for 12-18 months
▫ Symptomatic treatment
▫ Immunomodulators : levamisole
▫ Corticosteroids and γ-globulins
▫ IV fluids : dehydration
▫ Strict asepsis : prevent infection

20. • Terminal stage
▫ If the patient has entered the precoma or coma stage
 Protein intake should be reduced
 Administration of neomycin by gavage and 20% IV glucose drip
are helpful
 Oxygen administration
 Exchange transfusion

21. Surgical management
• No specific surgical correction
• Liver transplantation (ESLD)
• Sengstaken tube (portal hypertension and hematemesis)
• Portocaval anstomosis (portal hypertension and
hyperspenism)

22. Nursing management
• Nursing diagnosis
▫ Hyperthermia related to the inflammatory process in the liver
▫ Ineffective breathing pattern related to pressure on
diaphragm secondary to ascites
▫ Imbalanced nutrition less than body requirement related to
anorexia
▫ Diarrhoea or constipation related to acute abdominal
condition
▫ Parental anxiety related to management of the disease
condition.

23. Nursing interventions
• Provide symptomatic management
• Adequate rest
• Semi-fowlers position
• Daily abdominal girth measurement
• IV fluid as per order
• Small and frequent diet
• Protein rich food and massive doses of vitamin B6
• Aseptic precautions
• I&O chart
• Parental education
 Disease condition and management
 Avoid food rich in copper eg: drycnuts, chocolates, liver etc.
 Breast feeding and weaning at 6 months
 Milk should not boiled and stored in copper or copper alloy pots
 Reduce use of brass and copper vessels
 Use aluminum and steel utensil

24.  Food rich in tryptophan should be reduced Eg: milk, eggs, meat,
nuts, beans, fish and cheese
 Provide more vitamin B6 foods such as potato, banana, spinach,
soya bean, fruits, and vegetables (vitamin B6 helps to convert
tryptophan to niacin.